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39 Cards in this Set

  • Front
  • Back
CLL is defined as...
mature
<20% blasts
a lymphoma...
presents primarily with a tumourous tissue mass
a leukemia...
presents primarily with blood & BM involvement
What defines a population as lymphoid?
surface antigens
antigen receptors (B or T) gene rearrangement
What defines a lymphoid population as neoplastic?
population of clonal lymphocytes capable of formation of tumorous masses or invasive growth
What is an alternative definition for neoplastic lymphoid population?
clone of lymphocyte with aquired genetic lesions confering autonomy from normal regulatory controls
What is the demographics for CLL/SLL?
most common leukemia of US
M:F 2:1
>50 yo
Charactersitics of CLL
asymptomatic
hypogammaglobulinemia!!!
autoimmune phenomena
BM involved
Immunophenotype of CLL is
CD 5+
CD 19 + pan B Ag
CD 10 - CALLA
CD 23 +
FMC7 -
What are the major prognostic factors for CLL?
stage
karyotype
mutational status
Zap 70 expression
IgH genes in B cells somatic hypermutation
normal B cells get many random pt mutations in Ig variable region during passage through germinal centers to generate Ab diversisty
How are IgH gene Bcell lymphomas categorized?
pre germinal center
germinal center
post germinal genter
pre germinal center b-cells
no somatic hypermutation
Germinal center B-cells
ongoing somatic hypermutation
Post germinal center Bcells
stable pattern of somatic hypermutation
What is the main mutational status change in CLL?
IgV
what are the characteristics of unmutated IgV CLL
aggressive
poor prognosis
What does Igv coorelate with?
Zap 70 expression
Cd 38
What are the transformations seen in CLL?
prolymphocytoid
Ricthers syndrome
What is prolymphocytoid transformation?
gradual accumulation of prolymphocytes in blood
clonal evlolution
more aggressive
What is Ricther's syndrome?
pleomorphic large B cell lymphoma
abrupt change
poor prognosis
various genetic lesions
What is the pt profile for hairy cell leukemia?
middle aged guy iwth cytopenias & a big spleen
What is the main immunophenotype for hairy cell leukemia?
CD 103 +
What are the other immunophenotypes fdor hairy cell leukemaia?
CD 19 +
CD 20 +
CD 5 -
CD 10 -
Co expression CD 11 & 22
How do you treat hairy cell leukemia?
2-CDA
What is large granular lymphycyte leukemia?
expansion of clonal CD 8+ T cells
What is the patient profile for large granular lymphocyte leukemia?
~60
male slightly more than female
What are sx of large granular lymphocyte leukemia?
splenomegaly 50%
rare LA
RA in 30% pts
NEUTROPENIA
Prognosis of large granular lymphocytic leukemia?
stable clinical course
lacks destructive growth
What is sezary syndrome?
leukemic form of mycosis fungoides (indolent cutaneous Tcell lymphoma)
What are sx in Sezary syndrome?
erythroderma
LA
peripheral blood lymphocytosis
highly aggressive
What is the patient profile for sezary syndrome?
M>F
~50
What do cells of sezary syndrome look like?
ceribriform cells - grooves, clefts & infoldings making the cell look like a brain
What is the immunophenotype for sezary syndrome?
CD4+
What is adult T-cell leukemia assoicated with?
HTLV-1
Where do you see HTLV-1?
SW Japan & caribbean
What are the sx in adult T-cell leukemia?
LA, hepatosplenomegaly, skin infiltration, hypercalcemia
What do the cells of adult T-cell leukemia look like?
deep nucelar clefts - flower
What is the immunophenotype for adult t-cell leukemia?
CD 4+ (like SS)